Motor and cognitive deficits in the heterozygous leaner mouse, a Cav2.1 voltage-gated Ca2+ channel mutant.

摘要: The leaner mutation in mice affects the Ca(v)2.1 voltage-gated calcium channel alpha(1A)-subunit gene (Cacna1a), causing a reduction currents predominantly Purkinje cells. This causes severe progressive cerebellar ataxia, beginning around postnatal day 10, homozygous (tg(la)/tg(la)), while their heterozygous littermates (tg(la)/+) present no obvious behavioral deficits. In humans, mutations Cacna1a orthologous produce broad range of neurological manifestations. To evaluate phenotypic status tg(la)/+ animals, we assessed motor performance and cognition, at different ages, these mutant mice. We were able to observe age-dependent impairment cognitive tasks; balance learning deficits found demanding tasks on rotarod hanging wire test, spatial memory was observed Morris water maze. Progressive dysfunction escape reflexes, indicative impairment, also animals. Although not presenting major alterations, show